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Related to lamellar ichthyosis: congenital ichthyosiform erythroderma
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KEY WORDS: Lamellar ichthyosis, Rickets, Vitamin D deficiency.
Effective treatment of severe thermodysregulation by oral retinoids in a patient with recessive congenital lamellar ichthyosis.
Egor suffers from a painful and disfiguring skin condition called Lamellar ichthyosis - and no one in the over-stretched Zhdanovichi Orphanage in Minsk wanted to touch him for fear that they too would catch the congenital condition.
700 g); facial dysmorphia, brachycephaly, lamellar ichthyosis involving the whole integument, and pyodermic foci on scalp and buttocks; and polysplenia with a pancreas of normal size.
The two most common phenotypes are lamellar ichthyosis and non-bullous congenital erythroderma which account for 75% of cases and others like Sjogren-Larsson syndrome, Netherton syndrome, Gaucher disease etc account for 15% of the cases.
Oral retinoid (RO 10-9359) in children with lamellar ichthyosis, epidermal hyperkeratosis and symmetrical progressive erythrokeratodermia.